Pseudoangiomatous Stromal Hyperplasia of the Breast Presenting As Gigantomastia: Case Report
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CASE REPORT http://doi.org/10.29289/25945394202020200062 Pseudoangiomatous stromal hyperplasia of the breast presenting as gigantomastia: case report Juliana Pontes Farias1* , Humberto Espínola Guedes Neto1 , Ariano Brilhante Pegado Suassuna1 , Rafael Chaves Claudino de Queiroga1 , Arthur Antonino da Silva Nunes1 , Adriano de Lima Quirino1 , Thaíse Lopes Medeiros1 ABSTRACT Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a benign condition generally seen as an incidental finding of biopsies for other causes. In some rare cases, it can evolve with expressive growth of breast tissue, leading to large breasts. The present study presents a case report of a patient who started with breast hypertrophy during lactation, evolving with gigantomastia, and arrived at the office 3 years after gestation with breasts of 6.2 and 4.3 kg and growth of accessory breast and axillary lymph nodes. The patient was emaciated, with bodily pain and psychological distress. Computed tomography was performed with the finding of diffuse retroglandular nodules and axillary and mediastinal lymph node enlargement, without being able to rule out the lymphoproliferative process. Then, core biopsies were made in several places on the breast whose pathological examination revealed PASH samples. Bilateral mastectomy was performed as a surgical treatment, with resection of the left accessory breast and left axillary lymph node, and breast reconstruction, with placement of silicone prostheses and graft of the areola-papillary complex, together with a plastic surgeon. She evolved in the postoperative period with good healing of the surgical wound, weight gain, necrosis of the areola-papillary complex and with severe psychiatric disorder, which was treated appropriately by psychiatrists, with remission of symptoms. This case report presents a rare evolution of PASH with gigantomastia, bringing intense physical and psychological distress to the patient, whose treatment chosen was total surgical resection of the breast tissue and aesthetic reconstruction. KEYWORDS: breast neoplasms; breast implantation; lactation; mastectomy; pathology. INTRODUCTION CASE REPORT Pseudoangiomatous stromal hyperplasia (PASH) is a rare benign A 27-year-old woman was referred to the outpatient mastology condition of the breasts first described in 19861, most commonly service at the Hospital Universitário Lauro Wanderley (HULW), in found in women in pre- and perimenopause2 with findings on João Pessoa, Brazil, with the complaint of bilateral gigantomas- imaging tests not specific to this condition, requiring a correlation tia accompanied by weight loss. She was seen for the first time with the anatomopathological aspect for diagnostic confirma- in August 2019, reporting breast enlargement that started dur- tion3. PASH can coexist with other breast lesions or even mimic ing pregnancy (three years ago) and worsened during lactation. fibroadenoma and it can progress, in spite of its being a benign After the first consultation, the patient did not attend the disease4. The diagnosis of this condition usually occurs inciden- return visit and only in February 2020 did she return to the clinic tally during investigation with anatomopathological study of for further investigation. The breasts had almost doubled in size other benign or malignant diseases of the breast5. Treatment var- compared to the first consultation, with engorged vessels and ies depending on the presentation. areas with necrosis and suppuration (Figure 1). Diagnostic hypoth- The objective of this study was to report a case with atypical eses were raised for PASH and breast lymphoma, both rare condi- evolution and surgical treatment with bilateral mastectomy and tions. Malignant neoplasm was considered due to rapid growth, immediate reconstruction, since the patient presented a rare and the presence of axillary lymph node enlargement and signifi- diffuse PASH condition beginning in pregnancy with rapid and cant weight loss. Complementary investigation did not include bilateral breast enlargement. mammography due to the patient’s age and, mainly, due to the 1Hospital Universitário João Pessoa, Universidade Federal da Paraíba – João Pessoa (PB), Brazil. *Corresponding author: [email protected] Conflict of interests: nothing to declare. Received on: 10/04/2020. Accepted on: 11/09/2020. Mastology 2020;30:e20200062 1 Farias JP, Guedes Neto HE, Suassuna ABP, Queiroga RCC, Nunes AAS, Quirino AL, Medeiros TL impossibility of performing it in view of the technical limita- tions given the expressive size of the breasts. On the day of the consultation, a computed tomography (CT) scan of the chest was performed, with and without iodinated venous contrast, revealing an important volumetric increase in the breasts due to the multiple confluent nodular formations, predominantly retroglandular, which exhibited slight impregnation by means of contrast, of etiology indeterminate to the method, and it was not possible to discard a lymphoproliferative process. The pres- ence of multiple lymph node enlargement in axillary chains and in the internal chest wall bilaterally also stands out (Figures 2A and 2B). A laboratory screening with general exams and serology was requested to rule out associated conditions and to obtain the necessary exams for surgery. Fragments of the left breast were also collected by means Figure 1. (A) Front view of the breasts; (B) right breast in lateral of a core-biopsy for histopathological analysis. The result was view, with suppurative ulcer; (C) left breast in lateral view and released on February 10th, 2020, revealing breast tissue with accessory breast (photographs taken in February 2020). pseudoangiomatous stromal hyperplasia (morphological aspects Figure 2. (A) Chest computed tomography shows a volumetric increase in the breasts due to multiple confluent nodular formations, predominantly retroglandular; (B) chest computed tomography highlighting the presence of multiple lymph node enlargements in axillary chains and the presence of the accessory breast on the left; (C) surgical parts of the direct and left breast (top to bottom); (D) anatomopathological showing stromal pseudoangiomatous hyperplasia, nodular and diffuse forms; (E) lymph nodes with reactive lymphoid hyperplasia; (F) absence of signs of malignancies. 2 Mastology 2020;30:e20200062 Pseudoangiomatous stromal hyperplasia of the breast presenting as gigantomastia: case report consistent with the diffuse form of PASH) and absence of signs hyperplasia; skin without particularities; absence of signs of malignancy. of malignancy; On March 12th, 2020, the patient underwent bilateral total • Axillary lymph nodes on the left (dimensions = 2.5 × 2 × mastectomy and immediate breast reconstruction by the ser- 0.8 cm): lymph nodes with reactive lymphoid hyperplasia; vices of mastology and plastic surgery, respectively, at HULW. absence of signs of malignancy. Both breasts, the left accessory breast, and the left axillary lymph nodes were resected (Figures 2C and 3A). The areola-papillary In the first postoperative visit, the patient returned without complex was preserved and used as a graft in the reconstruc- complaints. On physical examination, significant edema was tion of the breasts, and silicone prostheses were implanted in a observed in the inframammary folds (Figure 3C). On the sec- submuscular position (Figure 3B). ond return, she presented remission of these findings and com- Surgical specimens were sent for histopathological study at plained of delusions, being referred to the psychiatric service, the Pathological Anatomy Laboratory (Laboratório de Anatomia where drug treatment was started. In the third consultation, Patológica – LAP) of the same hospital (Figures 2D, 2E, and 2F). she showed improvements in psychiatric symptoms and the The results, released on March 26th, 2020, were: removal of the surgical stitches was performed. Partial necrosis • Right breast (weight = 6,255 g; dimensions = 38 × 32 × 10 cm): of the areola-papillary complex grafts and progressive weight pseudoangiomatous stromal hyperplasia, nodular and gain, around 15 kg, were observed in relation to the beginning diffuse forms; skin without particularities; absence of signs of the follow-up (Figure 3D). The possibility of a corrective sur- of malignancy; gical procedure was offered, however, the patient expressed no • Left breast (weight = 4,295 g; dimensions: 29 × 27 × 12 cm): interest in performing a new surgery. pseudoangiomatous stromal hyperplasia, nodular and The study was carried out according to the ethical princi- diffuse; skin without particularities; absence of signs of ples of studies in human beings according to the Declaration of malignancy; Helsinki, with the approval of the Research Ethics Committee • Left accessory breast (dimensions = 11 × 8 × 5 cm): of Hospital Universitário Lauro Wanderley, with CAAE number ectopic breast tissue with pseudoangiomatous stromal 36548520.2.0000.5183. Figure 3. (A) marking and skin flaps after mastectomy; (B) frontal and lateral view of the immediate postoperative period of breast reconstruction after total mastectomy with resection of the left accessory breast; (C) postoperative period of April 2020, showing signs of partial necrosis of the areola-papillary complex grafts; (D) Late postoperative period of September 2020, showing good healing of the surgical wound, in addition to the patient’s weight gain. Mastology 2020;30:e20200062 3 Farias JP, Guedes Neto HE, Suassuna ABP, Queiroga RCC, Nunes AAS, Quirino